Vitamin Status in Children with Cystic Fibrosis Transmembrane Conductance Regulator Gene Mutation.

BACKGROUND: The issue of vitamin metabolism in children with cystic fibrosis screen positive, inconclusive diagnosis (CFSPID) is not well known. The aim of this study was to determine the status of vitamins A, D, E, and C in the blood of a group of children with CFSPID. MATERIAL AND METHODS: A total of 89 children were enrolled in the study (Me: 3.6 years, 52.8% boys), as follows: 28 with CFSPID, 31 with CF (cystic fibrosis), and 30 HC (healthy children). Their blood concentrations of vitamins A, D, E, and C, and their dietary intake of these vitamins were analysed in the study groups on the basis of a three-day food diary. RESULTS: The patients with CFSPID had significantly higher serum vitamin D (p = 0.01) and E (p = 0.04) concentrations, compared to the children with CF. None of the children with CFSPID revealed vitamin A or E deficiencies. Patients with CF had been consuming significantly higher vitamin D and E amounts (p = 0.01). The vitamin concentrations did not depend either on the pancreatic/liver function or on anthropometric parameters. In total, 32.14% of patients with CF did not cover the baseline recommended calorie intake, and 53.6% and 36% did not take the recommended vitamin E and vitamin A intake, respectively. CONCLUSION: Children with CF and CFSPID did not fully cover the dietary recommendations for vitamin supply, but vitamin deficiency was found only in CF.

Assessment of Liver Fibrosis with the Use of Elastography in Paediatric Patients with Diagnosed Cystic Fibrosis.

Background: Complications of cystic fibrosis-associated liver disease (CFLD) are a leading nonpulmonary cause of death. Noninvasive tests enabling early detection of liver changes, especially in children are sought. The aim of the study was to assess the scale of liver fibrosis with the use of elastography in paediatric patients with diagnosed cystic fibrosis (CF) and its comparison with other tests (APRI and Fibrotest). Methods: We examined 41 children, in the age range 2-21 years, with diagnosed CF. The analysis a included clinical picture, laboratory parameters of liver damage, and cholestasis. Aspartate aminotransferase-to-platelet ratio index (APRI) and Fibrotest were done in all patients. Liver stiffness measurements were acquired using shear-wave elastography (SWE). Results: CFLD was diagnosed in 16/41 patients (39%). Abnormal elastography was observed in 19/41 patients (46.3%), and in 5/41 (12.2%), the changes were advanced (F4). Abnormal elastography was observed in 12/16 (75%) of the patients with CFLD, and in 7/25 (28%), there were no lesions observed in the liver in the course of cystic fibrosis. In all patients with F4, we observed abnormal results of the APRI and Fibrotest. In most patients with small changes in elastography, we found normal results of the APRI and Fibrotest. Conclusion: Elastography seems to be a noninvasive examination useful in everyday clinical work in detecting early liver changes and monitoring of progression in paediatric patients with diagnosed cystic fibrosis, ahead of changes in laboratory tests. The cost-effectiveness of this test, the possibility of its repetition, and its availability are additional benefits.

Amount of Fibre in the Diet with Regard to Excessive Weight and Obesity among Children and Adolescents in Rural Communities.

One component of a correctly balanced diet is dietary fibre. Fibre acts protectively-it improves the functioning of the intestines, regulates the rhythm of bowel movements, inhibits the absorption of sugar and also lowers the level of cholesterol. The aim of the research was to determine the intake of fibre in relation to the occurrence of excessive weight and obesity among children and adolescents living in rural areas. The research was conducted using an authored questionnaire. The study questionnaire was completed by the study participants and their parents over 7 d. The anthropometric measurements were carried out on pupils in their underwear in conditions of privacy. Based on the results obtained, the BMI index was calculated for each pupil and then ranked according to WHO reference values. Among the pupils in the study group, the intake of fibre was at a very low level. The lowest amount of fibre in the diet was found among those with excessive weight and with obesity. Over 39% pupils never consumed wholegrain bread. Fruit and vegetables were consumed most seldom by pupils with excessive body weight. Knowledge about the lifestyles of children and adolescents is of crucial importance in taking multidirectional preventative actions to make changes to such lifestyles.

Assessment of Selected Parameters of Liver Fibrosis and Inflammation in Patients with Diagnosed Cystic Fibrosis.

Changes in the liver and bile ducts observed in patients diagnosed with cystic fibrosis result from inflammatory processes as well as fibrosis, remodeling, apoptosis, and cholestasis. As a consequence, portal hypertension, cirrhosis, and hepatic failure may develop. So far, the complexity of these processes has not been elucidated. Study Objectives. The aim of the study was to evaluate the selected parameters of hepatitis and fibrosis (Fibrotest, Actitest, and APRI) in patients diagnosed with cystic fibrosis. Material and Methods. The study included 79 patients with cystic fibrosis, aged 1 to 20 years (mean age 9.8 years), 49 girls (62%) and 30 boys (38%). The analysis involved the following: age, sex, clinical manifestations, laboratory tests evaluating pancreas function, parameters of liver damage, and cholestasis. Fibrotest, Actitest, and APRI were performed in all subjects. Results. Elevated parameters of hepatic cell damage (hypertransaminasemia) were found in 31/79 (39.2%) patients, while abnormal cholestasis parameters in 21/79 (26.6%). The abnormal results of Fibrotest were reported in 15% of patients (12/79), while of Actitest in 10% (8/79). In contrast, elevated APRI values were found in only 7.6% (6/79) of subjects. There was a statistically significant correlation between APRI and age (higher values were observed in younger children) and between Fibrotest and Actitest and pancreatic insufficiency (higher values were found in subjects without this abnormality). Moreover, Fibrotest values were significantly higher in girls. There was no correlation between Fibrotest, Actitest, and APRI values and the type of mutation. Conclusion. It appears that Fibrotest may be used as an early marker of liver fibrosis in patients with cystic fibrosis. Increased APRI values were only found in subjects with advanced hepatic lesions, most often in the form of portal hypertension.

Serum Level of D-Lactate in Patients with Cystic Fibrosis: Preliminary Data.

D-Lactate is produced by the intestinal biota and later absorbed into circulation. Some patients with cystic fibrosis (CF) develop exocrine pancreatic insufficiency that may disturb the gut microbiome and enhance the production of D-lactate. However, this concept has not been studied yet. The aim of the study was to assess D-lactate concentration in relation to the occurrence of clinical features, activity of CF, and diet composition in paediatric patients. Patients and Method. Serum concentrations of D-lactate were measured in 38 CF patients (19 girls and 19 boys) from 6 months to 18 years of age. The analysis included age, sex, clinical symptoms, diet (the variety and calorie needs), the laboratory tests for pancreatic efficiency (serum levels of albumin and glucose, faecal elastase activity, and faecal fat index) and faecal calprotectin (the marker of intestinal inflammation), and parameters of liver damage and of cholestasis (the activity of aminotransferases, γ-glutamyltransferase, level of bilirubin, and international normalized ratio). Results. The median level of D-lactate was 0.86 µg/ml (1Q-3Q: 0.48-2.03) and correlated with the CF severity in the Schwachman-Kulczycki score, parameters of pancreatic insufficiency, and the presence of intestinal inflammation. An increased level of D-lactate was observed in the subgroup with pancreas insufficiency (1.05 versus 0.73; p < 0.05), parallel with an elevated level of calprotectin (0.948 versus 0.755; p = 0.08). There was no relationship between energy consumption and diet composition and serum D-lactates. Conclusion. Serum D-lactate concentration in CF patients is a promising new marker of exocrine pancreatic insufficiency probably related to intestinal flora dysbiosis/overgrowth.

Serum Hepcidin Level as a Marker of Iron Status in Children with Cystic Fibrosis.

INTRODUCTION: Iron deficiency is common in patients with cystic fibrosis. Conventional iron status markers are often abnormal in patients with CF, reflecting inflammation and/or infection, rather than actual iron stores. The aim was to evaluate serum hepcidin levels against selected iron status markers, assuming that hepcidin may be a more sensitive indicator of iron management in patients with active inflammation, such as those with CF. MATERIAL AND METHODS: 46 children with cystic fibrosis and 31 healthy controls were enrolled. Hepcidin concentration was evaluated, along with the following other blood assays: full blood count, Fe, ferritin, transferrin, TIBC, liver markers, and CRP. RESULTS: Higher ferritin and CRP levels as well as lower TIBC levels significantly predicted hepcidin levels in the study group, control group, and the entire sample. There was no significant difference in hepcidin levels between the patients and controls. Children with exacerbations had significantly higher hepcidin levels than those with stable disease. These findings support the serum hepcidin level as useful in assessing iron status in children with cystic fibrosis. It may also be useful in early detection and monitoring of treatment of exacerbations.

Acute pancreatitis in children.

INTRODUCTION: The acute pancreatitis is a rare disease, but it has started to be diagnosed more often in children. AIM: The aim of the study was single-centre, retrospective analysis of the incidence, aetiology, and clinical course of acute pancreatitis in children. MATERIAL AND METHODS: We analysed the medical records of patients with acute pancreatitis hospitalised in the Gastroenterology Unit of the Paediatrics Department, Medical University of Silesia from Jan 2004 to Dec 2013. RESULTS: There were 76 cases of acute pancreatitis among 51 children (average age: 12.07 years) hospitalised in the Gastroenterology Unit between January 2004 and December 2013. The diagnosis of acute pancreatitis was performed on the basis of INSPIRE criteria and modified Atlanta classification. Patients were divided into groups: I - 1-12 years old, which included 20 (39.21%) children and II - 13-18 years old, with 31 (60.78%) children. The idiopathic aetiology was the most common cause of acute pancreatitis, occurring in 22 (43.1%) children, and in 15 cases the aetiology of the disease was biliary (29.4%). Genetically determined causes were diagnosed in 8 (15.7%) patients, the PRSS1 mutation in four patients, mutation in SPINK1 in 1 child, and CFTR gene mutation in 1 child. Two children simultaneously had two genes mutations (CFTR, SPINK1), and during the considered period had more than one episode of acute pancreatitis. CONCLUSIONS: Acute pancreatitis of idiopathic aetiology was most common among the examined children, and this should encourage the continued search for the causes of disease, especially genetic, and with particular emphasis on younger age group.

Serum levels of vascular endothelial growth factor and basic fibroblast growth factor in children with brain tumors.

BACKGROUND: Angiogenesis is the process of new vessel formation originating from the existing vascular network. It plays an important role in the growth and spread of malignancies, including brain tumors. The process of angiogenesis is characterized by increased expression of vascular endothelial growth factor (VEGF) and basic fibroblast growth factor (bFGF), and by the release of their soluble forms into circulation. OBJECTIVES: The aim of the study was to evaluate serum levels of VEGF and bFGF in children with malignant and benign brain tumors. MATERIAL AND METHODS: The study group (group N) included 106 children diagnosed with brain tumors. The children in group N were classified according to tumor pathology into 3 subgroups: N1 (n = 63): patients with malignant tumors, excluding anaplastic astrocytoma (AA) and glioblastoma multiforme (GBM); N2 (n = 25): patients with benign tumors; and N3 (n = 18): patients with high grade gliomas (AA and GBM). VEGF and bFGF were determined by ELISA in blood samples before the initiation of chemotherapy. VEGF and bFGF levels were compared within the subgroups in relation to tumor grading and the extent of surgery. RESULTS: The median VEGF in patients with brain tumors was significantly higher than in the control group. The median levels of VEGF and bFGF in subgroup N1 were significantly higher than in the control group. The differences in VEGF and bFGF concentrations between the subgroups in relation to the extent of tumor resection were not significant. CONCLUSIONS: Significantly higher plasma VEGF levels in children with brain neoplasms may reflect enhanced angiogenesis in the tumors.

The concentration of calprotectin in the stools of children with diagnosed cystic fibrosis.

INTRODUCTION: Calprotectin is a protein that plays a regulatory role in inflammatory reactions as an antibacterial and antiproliferative factor. AIM: To assess the concentration of calprotectin in the stools of patients with diagnosed cystic fibrosis. MATERIAL AND METHODS: Forty-one patients were included in the study, 24 boys and 17 girls, aged from 7 weeks to 18 years. The concentration of calprotectin in stools was assessed with the ELISA method. The analysis included clinical symptoms and the results of laboratory tests and the type of mutation. RESULTS: An elevated level of calprotectin in the stool was observed in 4/41 (9.7%) patients, mainly in older children, and mainly delta F508/deltaF508 mutation. The correlation between the concentration of calprotectin and clinical symptoms, age, increased indicators of an inflammatory process, levels of protein and aminotransferases in blood serum and the values of acid steatocrit of the stool was not proven. CONCLUSIONS: High concentrations of calprotectin in the stools of children with diagnosed cystic fibrosis do not correlate with the level of advancement of lesions within the gastrointestinal tract. Elevated concentrations of calprotectin in the stools of patients with cystic fibrosis may indicate inflammation of intestine and should be further scrutinised.

Eosinophilic colitis in children.

INTRODUCTION: Eosinophilic colitis, which is a rare form of eosinophilic gastrointestinal diseases, occurs as primary and secondary allergic eosinophilic colitis of the gastrointestinal tract infection, inflammatory bowel disease, celiac disease, and vasculitis. The diagnosis is based on a significant amount of eosinophils in the inflammatory infiltrate of the colon wall. AIM: To analyze the clinical picture taking into account comorbidities and endoscopic picture in children with eosinophilic colitis. MATERIAL AND METHODS: The test group consisted of 43 children, the average age - 12.1 years diagnosed with eosinophilic colitis (according to the Whitington scale) hospitalized in the Gastroenterology Unit, Department of Pediatrics of the Medical University of Silesia in Katowice. Testing for food allergies, celiac disease, inflammatory bowel disease, gastrointestinal diseases and parasitic diseases was performed in the group of children and the analysis concerned the intensity of eosinophilic infiltration of the colon mucosa with the severity of clinical symptoms, endoscopic picture, the presence of inflammatory bowel disease, and food allergy. RESULTS: Half of the tested children suffered from isolated eosinophilic colitis but the rest of them had eosinophilic infiltrate with inflammatory bowel disease more often, however, the Crohn's disease. The endoscopic image was uncharacteristic, and grade III in the Whitington scale was predominant in the histopathological examination, in most cases located in the entire large intestine. The higher level of total IgE was found in less than half of the patients and it did not correlate with the severity of eosinophilic infiltration. It was shown that the severity of eosinophilic infiltration correlated with exacerbation of clinical symptoms, endoscopic image, and the presence of inflammatory bowel disease. The higher level of antibodies of ASCA and ANCA was found in approximately 20% of the children with isolated eosinophilic colitis and 63% of children with Crohn's disease. CONCLUSIONS: The higher concentration of total IgE in less than half of the patients with eosinophilic colitis indicates the need for improving allergy diagnosis also in terms of IgE-independent allergy. The presence of higher levels of antibodies of ASCA and ANCA in some of the patients with isolated eosinophilic colitis indicates the need for further observation for the occurrence of inflammatory bowel disease.

Renal function in children treated for central nervous system malignancies.

AIM: The aim of the study was to evaluate renal function and to assess the usefulness of the following nephrotoxicity markers: cystatin C (CYS C), beta-2 microglobulin (B2MG) and neutrophil gelatinase-associated lipocalin (NGAL) in 38 (18 girls, 20 boys) children previously treated for central nervous system malignancy. MATERIAL: Median age at evaluation was 13.7 years (range 2.1-22 years). The mean follow-up time after the completion of chemotherapy was 3.2 years (range 0.16-6.5 years). RESULTS: Subclinical chronic kidney disease (estimated glomerular filtration rate: eGFR 90-60 ml/min/1.73 m(2)) was found in 22 patients (58 %), while renal insufficiency (eGFR 30-60 ml/min/1.73 m(2)) was found in six children (16 %). It has been demonstrated statistically significant negative correlation between the eGFR and cystatin C concentration (p < 0.0001) and eGFR and beta-2 microglobulin concentration (p < 0.02). Conversely, there was no correlation between eGFR and NGAL. Thirteen children (34 %) developed drug-induced tubulopathy: decreased tubular reabsorption of phosphate (TRP) and renal tubular threshold for phosphate (Tmp/GFR). CONCLUSION: Children treated for CNS tumours often develop drug-induced chronic renal disease, involving the glomeruli and/or renal tubules. Cystatin C and beta-2 microglobulin seemed to be good markers for chronic kidney damage in these patients, which is probably not true for NGAL.

Inflammatory Myofibroblastic Tumor of the Heart in the Infant: Review of the Literature.

Primary heart tumors are extremely rare, constituting approximately 0.02% of all malignancies. Inflammatory myofibroblastic tumor (IMT) constitutes <5% of primary heart tumors. Until now, IMT of the heart has been described in 21 infants below 1 year of age. Its etiology remains unknown. IMT usually develops within the right atrial and ventricular endocardium. The main clinical symptoms reported in the affected infants involved increasing respiratory failure, cyanosis, and heart murmurs. Histopathologically, IMT is characterized by the myofibroblast proliferation with inflammatory infiltrates composed of plasmocytes, lymphocytes, and histiocytes. Tumor resection is the treatment of choice in IMT. Such tumor location is associated with the high risk of perioperative failure. Steroid therapy and chemotherapy is reported in the literature as a nonsurgical treatment alternative. Here, we present a review of clinical symptoms, diagnostic and treatment options, based on published case reports of IMT in infants, including our 11-month-old patient with IMT located within the pericardium.

Lactose Intolerance in Children with IgE-Dependent Allergy to Milk Proteins.

The aim of this study was to assess the frequency of the occurrence of lactose intolerance in children with an IgE-dependent allergy to cow's milk. The study group consisted of 48 children diagnosed with IgE-dependent allergy to cow's milk proteins (group I). The control group (group II) included 40 children, in a similar age range and with normal architecture of the mucosa of the small intestine, with excluded food allergy. All patients underwent gastroduodenoscopy with biopsy, in which the activity of lactase was determined. The average activities of lactase in the group of patients with allergy were lower than in group II and amounted to 5.6 and 8.64 U/1 g. Lactase deficiency was observed in 6/48 patients in the group of patients with allergy. In group II, lactase deficiency was observed in 20%, which corresponds to the frequency of lactase intolerance in Poland. Lower activity of lactase was statistically significantly more common in older children. A reduced activity of lactase was more frequent in children with atrophy of the intestinal villi; however, that difference was not statistically significant. Lactose intolerance was less frequent in children with a diagnosed IgE-dependent milk proteins allergy. Due to the positive role of lactose in the diet, it seems that including a lactose-free formula in patients with diagnosed IgE-dependent allergy to milk is not necessary.

Diagnostic problems in cystic fibrosis - specific characteristics of a group of infants and young children diagnosed positive through neonatal screening, in whom cystic fibrosis had not been diagnosed.

INTRODUCTION: Neonatal cystic fibrosis screening contributes to an early diagnosis of cystic fibrosis and to implementing appropriate therapeutic management. Long-standing screening tests have made it possible to identify a group of newborns in whom the diagnosis was ambiguous and required further specialised tests. AIM: The aim is to present cases of patients with a positive result of newborn screening for cystic fibrosis who were found to be carriers of the mutation in both alleles, however the lack of clinical symptoms and correct sweat testing values did not lead doctors to diagnosing cystic fibrosis and by the same token implementing the treatment. MATERIAL AND METHODS: The analysis encompassed a group of 22 infants and children 3 months to 3 years of age, in whom, in spite of a positive result of newborn screening for cystic fibrosis and the presence of 2 mutations in the CFTR gene, the diagnosis of cystic fibrosis was not made, and appropriate treatment was not administered because of diagnostic doubts (due to correct concentration of chlorides in sweat, correct IRT level and lack of clinical signs of cystic fibrosis). The control group consisted of 55 children treated in our centre, in whom neonatal screening for cystic fibrosis was positive and the diagnosis was confirmed by genetic testing, sweat chloride testing and IRT concentration. RESULTS: There were no differences in birth body weight between the groups. The differences in chlorideion levels in sweat secretion tests and mean IRT values were statistically significant and were: 97.5 for the control group and 26.4 for the test group. At the present time there are no clinical symptoms to give a diagnosis of cystic fibrosis and start treatment in the test group. CONCLUSIONS: Newborn screening contributes not only to an early diagnosis of cystic fibrosis but also to CFTR-related metabolic syndromes (CRMS), which is a phenomenon requiring further observation. This fact constitutes a definite psychological problem for the parents of these patients. .

Does the mutation of the SERPINA1 gene contribute to liver damage and cholestasis in patients with diagnosed cystic fibrosis? preliminary study.

UNLABELLED: Mutation of the SERPINA1 gene is present in about 2% of patients with cystic fibrosis but is more common and accounts for about 5% in patients with cystic fibrosis and co-existing liver lesions. The SERPINA1 gene is responsible for the synthesis of a serine protease inhibitor. The protein related with this gene is accumulated within the endoplasmic reticulum of hepatocytes causing their damage, inflammation and cirrhosis. The aim was to assess the presumable effect of the SERPINA1 mutation gene in patients with diagnosed cystic fibrosis on damage to the liver and/or cholestasis. MATERIAL AND METHOD: The analysis included 30 children, 13 girls (43.3%) and 17 boys (56.6%), aged from 6 months to 18 years (the average age was 5.5 years) with diagnosed cystic fibrosis. All the patients have undergone a genetic test of the mutation of the SERPINA1 gene. The analysis included age, sex, clinical symptoms, type of mutation of the CFTR protein, abnormalities in laboratory tests (the activity of aminotransferases, GGTP, alkaline phosphatase , protein, the indicator of acid steatocrit, the rate of APRI) and abdominal ultrasonography. RESULTS: Symptoms of damaged liver were concluded in 9/30 patients (30%) with diagnosed cystic fibrosis. Most commonly observed were increased activities of aminotransferases in 9/30 patients (30%) and of gamma glutamyl transferase in 6/30 (20%) of the assessed patients. In 4/30 patients the abdominal ultrasonography revealed an enlarged liver and increased echogenicity. Mutation within the SERPINA1 gene was observed only in 1/30 patients (3.3%) with diagnosed cystic fibrosis. As far as the patient is concerned, currently the activities of aminotransferases, GGTP and AF are normal, but there has been a considerable increase in the intensity of symptoms from the respiratory system. No corelation between the mutation of the SERPINA1 gene and clinical symptoms, type of mutation of the CFTR protein, laboratory results of the functions and damage to the liver and the abdominal ultrasonography was observed. CONCLUSIONS: We did not find a more frequent occurrence of the SERPINA1 gene mutation in children with cystic fibrosis and coexisting features of damaged liver and cholestasis. The obtained results suggest the contribution of other than SERPINA1 gene mutation factors responsible for the development of changes in the liver in patients diagnosed with cystic fibrosis. The studies on the subject should be extended and performed on a larger group of patients. .

Disaccharidase activity in children with inflammatory bowel disease.

BACKGROUND/AIMS: The etiopathogenesis of inflammatory bowel disease (IBD) is multifactorial and not well explained. Environmental, genetic, and dietary factors play an important role. The aim of the study was the evaluation of lactase, saccharase, and maltase activity in patients with IBD. MATERIALS AND METHODS: The study comprised 65 children, aged 3-18 years. During a routine endoscopy, we took biopsies from the descending part of the duodenum. In these biopsies, we determined disaccharidase activity using Dahlquist's method. RESULTS: Decreased lactase activity in the biopsies taken from the small intestine mucosa was most frequently observed in patients with Crohn's disease (5/15-33%) and least frequently seen in children with lymphocytic colitis (in 1/10-10%). The lowest mean values of lactase activity were found in the children with Crohn's disease and ulcerative colitis (1.7-2.5 U/1 g). Decreased saccharase activity in the biopsies obtained from the small intestine mucosa was most frequently observed in patients with lymphocytic colitis (in 5/10-50%) and ulcerative colitis (9/20-45%) and least frequently seen in children with non-specific undetermined colitis (in 7/20-35%). Decreased maltase activity in the small bowel mucosa was the most frequently observed in patients with Crohn's disease (in 5/15-33%) and least frequently seen in children with ulcerative colitis (in 3/20-15%). The lowest mean values of maltase activity were found in the children with Crohn's disease (5.4 U/1 g). CONCLUSION: Therefore, it seems reasonable to perform diagnostic examinations aimed at lactose, saccharose, and maltose intolerance and to initiate a dietary regimen in children with IBD.

Leptin concentration and nutritional status in the course of treatment in children with brain tumours--preliminary report.

PURPOSE: To assess the nutritional status in children with central nervous system (CNS) tumours, including concentration of leptin, the neuropeptide responsible for regulation of energetic homeostasis in an organism. METHOD: The studied group comprised 44 children with brain tumours, aged (4.02-18.7). In all children during the whole therapy (from the start to the period of 1 year and more after the end of therapy), a number of standard deviations (SDs) for the body mass index (SDS BMI) was derived from anthropometric measurements. Concentrations of leptin were assayed simultaneously. RESULT: The lowest values of the anthropometric indices were found in children during the maintenance therapy. Concentrations of leptin in patients with malignant CNS tumours and significant undernutrition were slightly greater as compared to patients presenting normal nutritional status; however, without statistical significance. CONCLUSION: In children with tumours of the central nervous system, there are quantitative disorders of the nutritional status which correlate with the period of the treatment. The most significant disorders in the nutritional status are observed during maintenance chemotherapy. There was no statistically significant correlation between the concentration of leptin and nutritional status in children with malignant brain tumours during the course of treatment and after its completion.

Expression of myeloid antigens on lymphoblast surface in childhood acute lymphoblastic leukemia at diagnosis and its effect on early response to treatment: a preliminary report.

Immunodiagnosis of acute lymphoblastic leukemia (ALL) is based on the assessment of surface antigens. There are also cases in which both lymphoid and myeloid antigens can be found on the surface of lymphoblasts. The purpose of our research was to assess the expression of myeloid and lymphoblastic antigens in children with ALL, and to determine the impact of surface antigens on early response to treatment. 58 children [33 girls (56.9 %), 25 boys (43.2 %)] with ALL were studied. Response to treatment was assessed on days 8, 15, and 33. Univariate logistic regression analysis of the effect of myeloid antigens (MyAg) on response to treatment on days 8 and 33 revealed expression of any MyAg on lymphoblast surface as a factor associated with poor response to treatment. The multivariate logistic regression analysis of treatment response on day 33, showed that the expression of CD13 antigen on lymphoblast surface is a key factor affecting delayed remission (p = 0.03; odds ratio 0.12; 95 % CI 0.01-0.81). The expression of MyAg in childhood ALL adversely affects early response to treatment. The expression of CD13 antigen on day 33 is a key factor affecting complete remission in ALL patients.

Aetiopathogenesis of liver changes in the course of cystic fibrosis, considering disturbances of the bile acid profile as well as genetic and immunological factors.

Liver changes observed in the course of cystic fibrosis comprise a group of complex processes of fibrosis, inflammation, remodelling, apoptosis and cholestasis as a result of abnormal functioning of the cystic fibrosis transmembrane conductance regulator (CFTR) protein, immunological reactions and response to oxidation stress. Liver lesions are only observed in 5-20% of patients with diagnosed cystic fibrosis; however, they increase mortality, reduce the lifespan and deteriorate the quality of life. Liver diseases are the most common extrapulmonary causes of death in patients with cystic fibrosis. The aim of the study was to analyse the hitherto performed studies on the aetiopathogenesis of liver changes in the course of cystic fibrosis considering disturbances of the bile acid profile as well as genetic and immunological factors.

Usefulness of faecal calprotectin measurement in children with various types of inflammatory bowel disease.

INTRODUCTION: The aim of the study was to assess the usefulness of the FC measurement in children with various types of IBD and relation to the disease activity. PATIENTS AND METHODS: 91 patients (49 boys: 53.85% and 42 girls: 46.15%, mean age: 13.38 years, range 6-18 years) were included in the analysis. Patients were divided into the groups: B1-24 children with CD, B2-16 patients with UC, and a group comprising 31 children with other types of colitis; the control group (K) comprised 20 healthy children. FC was assayed by ELISA method, using Phical test (Calpro). RESULTS: The mean faecal calprotectin concentrations were higher in children with CD and UC as compared to healthy controls, patients with eosinophilic, lymphocytic, and nonspecific colitis. A positive correlation was observed between FC concentrations and the disease activity (the PCDAI scale, the Truelove-Witts Scale, and the endoscopic Rachmilewitz Index). CONCLUSION: It seems that the FC concentrations can be a useful, safe, and noninvasive test in children suspected for IBD, since FC concentration is higher in children with CD and UC than in patients with other inflammatory diseases.